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Oral acitretin treatment in severe congenital ichthyosis of the neonate.

Two newborn infants with ichthyosis, one with lamellar ichthyosis and one with nonbullous ichthyosis form erythroderma, who presented at birth with a collodion baby appearance, were treated with acitretin (1 mg/kg/day). Clinical improvement was achieved shortly after treatment. The second case received oral retinoid for 3.5 months and was followed for nine months. The result was excellent. The treatment resulted in a satisfactory improvement in the skin condition of the first case. The tolerance to the drug was good. Side effects were not observed. It was concluded that early management of severe ichthyosis cases could prevent life-threatening events such as hyperthermia, disturbance in electrolyte and fluid balance, and infection.

Acitretin↗

[Microanatomy study on sinus triangle region].

OBJECTIVES: To explore shape and position relationship of the structures with in sinus triangle region, and provide the anatomic foundation for extended presigmoid operative approach. METHODS: Ten cadaveric heads and 15 skull-base specimens fixed with 10% formalin, with aid of operative microscope, the shape and relations of structures with in the sinus triangle region were observed and microphotographed. Four cadaveric heads were sectioned in different plane using collodion embedding process. RESULTS: Jugular blub (JB) had high -placed jugular blub (HJB), normal jugular blub (NJB) and low-placed jugular blub (LJB). The incidence of HJB was 8% and 18% for the left and right sides (P < 0.01). The JB dome can extend upward the hypotympanic cavity and the posterior wall of the internal acoustic meatus. The incidence of NJB and LJB was 36% and 30%, and 6% and 2% respectively. The level distance between the posterior semicircular canal and petrosal posterior surface was (4.1 +/- 1.1) mm. The distance between the JB and vertical part of the facial nerve was (3.2 +/- 0.5) mm and (2.0 +/- 0.4) mm. The distance between the JB and posterior margin of the internal acoustic port was (7.5 +/- 1.7) mm and (4.0 +/- 1.0) mm. CONCLUSIONS: Knowing the shape and position relationship of structures in the sinus triangle region is favorable for preserving the important structures in extended presigmoid operative approach.

Autopsy↗

[Trichothiodystrophy and congenital heart disease in two sisters].

INTRODUCTION: Trichothiodystrophy refers to a heterogeneous group of autosomal recessive genodermatosis of unknown etiology. Patients with trichothiodystrophy have alopecia with typical hair dysplasia ("tiger-tail"), and abnormally low sulfur content. Numerous unrelated neuroectodermal defects have been observed in trichothiodystrophy. We report here trichothiodystrophy and congenital heart disease in two sisters. CASES REPORT: Two sisters were born as collodion babies and presented ichthyosis and alopecia. The diagnosis of trichothiodystrophy was confirmed by polarizing microscopy of hair and low sulfur content. Both had congenital heart disease. DISCUSSION: Cardiovascular defects have rarely been reported in trichothiodystrophy. The association trichothiodystrophy - congenital heart disease has never been described. The low incidence of both conditions suggests that these abnormalities are linked etiologically rather than by chance. Abnormal Notch signaling, or contiguous gene deletion syndrome could lead to combined phenotype as observed in our family.

Alopecia↗

The clinical spectrum of congenital ichthyosis in Sweden: a review of 127 cases.

Congenital ichthyosis comprises a rare group of usually monogenetic diseases that present at birth as a collodion phenotype or as variable degrees of ichtHyosiform erythroderma, with or without superficial blisters. Depending on which gene mutation causes the disease, the skin problems later in life may range from a severe lamellar or bullous ichthyosis to mild or only focally expressed hyperkeratotic lesions. It is obviously important, but sometimes painstakingly difficult, to make a correct diagnosis already in infancy. Fortunately, recent advances in our understanding of the molecular genetics of ichthyosis have led to several new diagnostic tools that are continuously being updated. Based on this development, and on our own 5 years of experience in a national genodermatosis centre, we describe 127 cases of congenital ichthyosis examined in childhood or adulthood. Applying a combination of phenotypic and genotypic criteria, the patients were classified into three main groups: 1) Bullous ichthyosis (epidermolytic hyperkeratosis) and related disorders due to keratin mutations (n = 21); 2) Non-bullous ichthyosiform erythroderma and lamellar ichthyosis mainly due to transglutaminase 1 mutations (n = 80); 3) Syndromic ichthyosis, i.e. systemic (multi-organ) diseases due to many different causes (n = 26). Each group could be further stratified into 4-11 entities using mutation analysis, electron microscopy of epidermis and various other techniques. Our findings are discussed in relation to recent data in the literature emphasizing the clinical usefulness of various diagnostic procedures for ichthyosis.

Adult↗

[Goyrand J.G.B (1803-1866), surgeon and academician from Aix en Provence].

The authors present Jean-Gaspard-Blaise Goyrand, from Aix, who is not simply remembered for his description of the wrist fracture which has retained his name. His theses concerning permanent retraction of the fingers, presented to the Royal Academy of Medicine in 1833 and 1834, were contrary to those proposed by Dupuytren and proved to be completely accurate. All of Goyrand's work was edited by Masson in 1870, including a superb case report of giant elephantiasis of the penis and scrotum, a study on cleft lip and the technique of using collodion bands to close large wounds [corrected].

France↗

Cantharidin treatment of digital and periungual warts.

Seventy-six digital and periungual warts in 40 patients were treated topically with cantharidin, a potent blistering agent. The material, dissolved in equal parts of acetone and collodion, was applied directly to the warts. Occlusion facilitated blistering. No pretreatment was required. The warts were re-treated at weekly intervals until clinically cured.Fifty-six per cent of digital warts and 33 per cent of periungual warts cleared after a single application of cantharidin. Few required more than three treatments. Observation was continued for more than six months in more than half of the cases. Cure was lasting in about 70 per cent of the cases in which the long term result was known. Cantharidin ranks with liquid nitrogen in effectiveness, but it is painless to apply and does not cause scarring. For these reasons it is especially useful in children.The main disadvantage is pain and tenderness at the treated site for two to four days in some patients. This can be avoided by careful application of the drug. Occasionally new warts appear at the edge of the cantharidin blister. They are best treated by curettage and desiccation.

Blister↗

[Round window area temporal bone section versus CT].

OBJECTIVE: To locate round window area and its related structure on auris transection and CT for anatomical evidence of image diagnosis and clinical operation. METHODS: Fifteen normal head specimen fixed with 10% dehyde were scanned by high-resolution computed tomography on canthomeatal line. CT image (depth 1.00 mm, thick 1.00 mm) was obtained. Temporal bone-centered tissues were taken, decalcified, desiccated and socked with collodion, then embedded and made into sequential transactions (thick 1.00 mm). Lower surface of section was observed by both naked eyes and microscope, then scanned and photographed. Versus CT image, auditory ossicle, osseous semicircular canals, vestibule, round window, niche, cochlea, pyramidal eminence, internal acoustic meatus and cochlear aqueduct were identified respectively. RESULTS: There were 18-22 layers of temporal transection on CT image. Round window and round window niche always appeared on the 10th layer (R) and the 11th layer (L). The mean of the depth of anterior wall was 0.92 mm (R) and 0.90 mm (L), and depth 1.89 mm (R) and 2.04 mm (L). The average distance from niche to jugular fossa wall was 2.10 mm (R) and 2.39 mm (L). No significant difference among of thickness, depth and distance from niche to jugular fossa wall. CONCLUSIONS: Temporal bone transection specimen had a clear picture of anatomical position between round window area and its related structure. Versus CT, the result contributed to image diagnosis and operation on auris diseases.

Adult↗

[Histopathology study of supraglottic carcinoma with different vocal cord movement].

OBJECTIVE: To explore the histopathology characteristics of supraglottic carcinoma with different vocal cord movement, and to find the theory basis for partial laryngectomy. METHOD: Eighty-four cases of supraglottic laryngeal carcinoma specimen were imbedded in collodion, and then make whole-organ serial section for HE staining, observing under light microscope. RESULT: For the vocal cord fixation, the rate of invasion to arytenoids, to infra-paraglottic space and to both arytenoids and infra-paraglottic space was 46.2%, 15.4%, and 38.5%, respectively; For the vocal cord limitation, the rate of invasion to arytenoids, to infra-paraglottic space and to both arytenoids and infra-paraglottic space was 46.7%, 6.7%, and 13.3%, respectively; For the vocal cord with good movement, no cases occurred for the invasion to the infra-paraglottic space or arytenoids. The difference was significant between them (P<0.05). There was close relationship between the tumor invasion to infra-paraglottic space with to the ventricle of larynx: for the vocal cord limitation and fixation, the ratio of the tumor invading the infra-paraglottic space to the ventricle of larynx is 40.0% and 73.7%, respectively, the difference was significant between them (P<0.05). CONCLUSION: The invasion of supra-glottic carcinoma to the arytenoids or infra-paraglottic space is the main cause of the vocal cord limitation and fixation. We can do the operation of partial laryngectomy more precisely by observing the movement of the vocal cord and the invasion to laryngeal ventricle.

Adult↗

Lamellar ichthyosis.

A 6-year-old African boy with a history of a collodion membrane presented with scale in a generalized distribution and flexural accentuation. Large, brown, polygonal scales were present on the forehead, lateral aspects of the face, and extremities. The nature of the scales and the lack of erythroderma in this patient are consistent with a mild form of lamellar ichthyosis (LI). LI and nonbullous congenital ichthyosiform erythroderma (NBCIE) represent phenotypes at the poles of the autosomal recessive ichthyosis spectrum. Mutations in genes encoding transglutaminase 1 (TGM1), the ABCA12 transporter (ABCA12), ichthyin, lipoxygenase 3 (ALOXE3), and 12(R)-lipoxygenase (ALOX12B) have been shown to underlie both NBCIE and LI.

Child↗

Electron microscopic study of Bacteroides nodosus pili and associated structures.

Surface structures of Bacteroides nodosus were examined by electron microscopy. Collodion film and chrome shadowing were used for maximizing the visualization of B nodosus pili and ring structures. The existence of B nodosus pili in foot rot lesions was confirmed. Contrary to previous reports, it was found that B nodosus pili production can be retained through serial broth transfer under certain conditions. Capsule production by B nodosus was irregular in that it could be either lacking or variable in thickness. A bacteriophage capable of infecting B nodosus also was detected.

Animals↗

[Anatomical and computed tomographic analysis of the interaction between uncinate process and agger nasi cells].

OBJECTIVE: To investigate the anatomical interaction between uncinate process and agger nasi cell to better understand the anatomy of the frontal sinus drainage pathway by endoscopy, spiral computed tomography (CT) and sectioning. METHODS: Twenty-one skeletal skulls (forty-two sides) and one cadaver head (two sides) were studied by spiral CT together with endoscopy and collodion embedded thin sectioning at coronal plane. The sections with the thickness of 100 microm were stained with hemotoxylin and eosin. RESULTS: Under endoscopy, a leaflet of bone to the middle turbinate, which is given off by uncinate process, forms the anterior insertion of the middle turbinate onto the lateral nasal wall. The middle portion of the uncinate process attached to the frontal process of the maxilla in all of the skeletal nasal cavities, as well as the lacrimal bone in 78.6% of the skeletal nasal cavities. On CT scans, the agger nasi cell is present in 90.5% of the skeletal nasal cavities. While the lateral wall of the agger nasi cell is formed by lacrimal bone, the medial wall of the agger nasi cell is formed by uncinate process. And the anterior wall is formed by the frontal process of the maxilla. The superior portion of the uncinate process forms the medial, posterior and top wall of the agger nasi cells. The superior portion of the uncinate extends into the frontal recess and may insert into lamina papyracea (33.3%), skull base (9.5%), middle turbinate, combination of these (57.2%). CONCLUSIONS: The agger nasi cell is the key that unlocks the frontal recess.

Adult↗

Ichthyoses: differential diagnosis and molecular genetics.

Ichthyoses are a heterogeneous group of cornification disorders characterized by a generalized scaling of the skin. Common types such as ichthyosis vulgaris and X-linked recessive ichthyosis manifest after birth. In contrast, rare congenital ichthyoses are inherited diseases, which at birth typically present collodion membranes or ichthyosiform erythroderma. Syndromic ichthyoses display a variety of outstanding associated non-cutaneous symptoms. Because of their rarity these disorders often pose a diagnostic challenge for the clinician. This review discusses a broad spectrum of 13 isolated types of ichthyoses, 11 different syndromes with associated ichthyosis and four related cornification disorders. The clinical, ultrastructural and biochemical characteristics are described along with the different molecular causes of ichthyosis. Special attention is given to lamellar ichthyosis and non-bullous congenital ichthyosiform erythroderma. The different pathomechanisms causing ichthyosis provide a fascinating insight into the role of various proteins, enzymes, lipids and metabolic pathways involved in terminal epidermal differentiation/keratinisation.

Diagnosis, Differential↗

Visual evoked potential in young adults: a normative study.

It is important to acquire adequate normative data of visual evoked potential before using it as a diagnostic tool. A study using visual evoked potential with pattern (VEP-P) was conducted in Twenty male and seven female young healthy subjects of 17-35 years age. Ag/Agcl electrodes anchored on scalp with collodion at O1-A1, O2-A2 (10-20 system), with transition skin to electrode impedence kept at less than 5 K ohms were connected to MEB 5200 Evoked potential Recorder (Nihon Kohden, Japan). The evoked responses to 256 visual stimuli were recorded using transient pattern reversal (checker size 32'), frequency 1/sec and contrast between black and white checks 67%. The responses were averaged by the computer and absolute peak latency values for P100 alongwith other positive and negative waves worked out. P100 latency of 95.37 +/- 6.85, amp. 6.4 +/- 2.38 for males and 91.07 +/- 49 msec, amp. 6.88 +/- 2.79 microv for females are being reported. The P100 latency values of present study are similar to those reported in age and sex matched subjects of the western world, indicating that there are no ethnic variations in P100 of VEP.

Adolescent↗

[Hemofiltration and hemoperfusion performed in series for the purpose of removing beta 2 microglobulin].

With the objective to develop most efficient method for Beta-2-microglobulin elimination, the authors, using extracorporeal circulation placed behind the haemofilter a capsule containing 70 g of collodion coated with activated medicinal charcoal. This combination was used in 3 haemofilters made from different membranes. The results showed polysulphon to be the highest efficiency membrane out of those tested for Beta-2 microglobulin elimination. In combination with haemoperfusion, its efficiency increases significantly. Even if haemoperfusion increases efficiency by considerable percentage, the Beta-2 microglobulin elimination using other types of membranes lasts long and the total clearance remains low.

Adult↗

Routine clinical applications of hemodialysis-hemoperfusion in chronic renal failure: case reports.

Five hemodialysis patients were treated with combined hemodialysis-hemoperfusion with their conventional hemodialyzer plus a 70-gram ultrathin collodion coated activated charcoal device for a total of 63 months. Indications for this therapy included pericarditis, peripheral neuropathy, clotting of conventional hemodialyzers and reduction of dialysis time and frequency. The outcome was beneficial in all cases and stable biochemical and hematological parameters were maintained. No increase in heparin requirements was noted and the therapy was thought to be cost-effective.

Adult↗

Liquid nitrogen and salicylic/lactic acid paint in the treatment of cutaneous warts in general practice.

Patients with common hand warts and simple plantar warts attending a general practice wart clinic in Northern Ireland were assigned to one of three treatment groups - liquid nitrogen applied weekly, daily application of wart paint (lactic acid one part, salicylic acid one part, collodion four parts), or a combination of the two. Combination therapy cured 87% of common hand warts over a six week period, and was significantly more effective than either agent used separately (P<0.05). The results for simple plantar warts were disappointing and no treatment regimen proved to be significantly better than any other. These treatments were cheap, compliance and tolerance were good, and the six month recurrence rate was minimal. Given the likelihood that warts will multiply if left untreated we would recommend early active treatment for this condition.

Adolescent↗

Lamellar ichthyosis with episodic psoriasiform reaction pattern.

The skin of a girl born with the typical appearance of "collodion baby," evolved into an exfoliative erythroderma that clinically was lamellar ichthyosis. However, biopsy specimens done in early infancy showed psoriasis. Over the ensuing sixteen years she has continued to have clinical lamellar ichthyosis with rare occasions of febrile episodes and superficial pustules. Some biopsy specimens have been diagnosed as showing lamellar ichthyosis, while others have again shown psoriasis.

Adolescent↗